[Dural Arteriovenous Fistula:Indications and Outcomes].

The diagnosis of dural arteriovenous fistulas(DAVF)has advanced with the development of imaging techniques. The indication for treatment of DAVF is generally based on classification according to the venous drainage pattern, which determines whether the presentation is benign or aggressive. In recent years, with the introduction of Onyx, transarterial embolization has been increasingly used, and outcomes have improved, although some conditions are more suitable for transvenous embolization. It is important to select an optimal approach based on location and angioarchitecture. Since DAVF is a rare vascular disease with limited evidence, further validation of clinical results is needed to provide more established treatment guidelines.

Plasma SPARC Elevation in Delayed Cerebral Ischemia After Aneurysmal Subarachnoid Hemorrhage.

Matricellular proteins have been implicated in pathologies after subarachnoid hemorrhage (SAH). To find a new therapeutic molecular target, the present study aimed to clarify the relationships between serially measured plasma levels of a matricellular protein, secreted protein acidic and rich in cysteine (SPARC), and delayed cerebral ischemia (DCI) in 117 consecutive aneurysmal SAH patients with admission World Federation of Neurological Surgeons (WFNS) grades I-III. DCI developed in 25 patients with higher incidences of past history of hypertension and dyslipidemia, preoperative WFNS grade III, modified Fisher grade 4, spinal drainage, and angiographic vasospasm. Plasma SPARC levels were increased after SAH, and significantly higher in patients with than without DCI at days 7-9, and in patients with VASOGRADE-Yellow compared with VASOGRADE-Green at days 1-3 and 7-9. However, there were no relationships between plasma SPARC levels and angiographic vasospasm. Receiver-operating characteristic curves differentiating DCI from no DCI determined the cut-off value of plasma SPARC ≥ 82.1 ng/ml at days 7 - 9 (sensitivity, 0.800; specificity, 0.533; and area under the curve, 0.708), which was found to be an independent determinant of DCI development in multivariate analyses. This is the first study to show that SPARC is upregulated in peripheral blood after SAH, and that SPARC may be involved in the development of DCI without angiographic vasospasm in a clinical setting.

Neonatal respiratory support related to lung function abnormalities in school-age children with bronchopulmonary dysplasia.

OBJECTIVE: To elucidate the relationship between abnormal lung function (LF) at school age and neonatal respiratory support in very low birth weight children with bronchopulmonary dysplasia (BPD). STUDY DESIGN: We retrospectively examined 78 BPD children whose LF was evaluated at 8-9 years. LF abnormalities were defined by reduced values of spirometric parameters. Adjusted odds ratios (aORs) for abnormal LF by the type and postmenstrual age (PMA) of respiratory support were calculated using logistic regression analysis after controlling perinatal factors. RESULTS: Overall, 24 (31%) patients had LF abnormalities. Antenatal steroid use was associated with a decreased risk of abnormal LF [aOR, 0.31; 95% CI, 0.09-0.92]. Requiring positive-pressure support at 37 weeks' PMA correlated with abnormal LF [aOR, 4.58; 95% CI, 1.15-21.90]; whereas only low-flow oxygen at any PMA did not. CONCLUSION: Requiring positive-pressure support at 37 weeks' PMA could be an indicator of abnormal LF at school age.

Genetic and clinical landscape of childhood cerebellar hypoplasia and atrophy.

PURPOSE: Cerebellar hypoplasia and atrophy (CBHA) in children is an extremely heterogeneous group of disorders, but few comprehensive genetic studies have been reported. Comprehensive genetic analysis of CBHA patients may help differentiating atrophy and hypoplasia and potentially improve their prognostic aspects. METHODS: Patients with CBHA in 176 families were genetically examined using exome sequencing. Patients with disease-causing variants were clinically evaluated. RESULTS: Disease-causing variants were identified in 96 of the 176 families (54.5%). After excluding 6 families, 48 patients from 42 families were categorized as having syndromic associations with CBHA, whereas the remaining 51 patients from 48 families had isolated CBHA. In 51 patients, 26 aberrant genes were identified, of which, 20 (76.9%) caused disease in 1 family each. The most prevalent genes were CACNA1A, ITPR1, and KIF1A. Of the 26 aberrant genes, 21 and 1 were functionally annotated to atrophy and hypoplasia, respectively. CBHA+S was more clinically severe than CBHA-S. Notably, ARG1 and FOLR1 variants were identified in 2 families, leading to medical treatments. CONCLUSION: A wide genetic and clinical diversity of CBHA was revealed through exome sequencing in this cohort, which highlights the importance of comprehensive genetic analyses. Furthermore, molecular-based treatment was available for 2 families.

Accelerated Cranioplasty with Perforator-preserved Split Flap Sandwiched Plate for Treatment of Infected Cranial Defects.

Large cranial vault defects are challenging to manage due to the need to balance infection control, skin coverage, and restoration of the protective mechanical rigidity of the skull while also ensuring good head and neck aesthetic results. Conventional-staged treatment requires a long time period that includes debridement and scalp skin defect coverage with flaps as a first step, followed by definitive plate reconstruction 3-9 months later after infection control and flap atrophy resolution. We report a case of successful early cranioplasty in a factory worker who developed a large full-thickness cranial skull defect following artificial dura infection. Reconstruction was performed in two stages using an anterolateral thigh (ALT) flap. In the first stage, the scalp defect was covered with an ALT flap to close the skin following debridement. In the second stage performed 6 weeks later, the ALT flap was split into adiposal and adipocutaneous flaps to sandwich a computer-aided design custom-made titanium plate with an opening for the perforator to complete the cranioplasty. The patient successfully returned to work without recurrence of infection with 1-year follow-up. We report this case to demonstrate the utility of adipocutaneous flap plate sandwiching techniques in providing well-vascularized cover for early definitive cranial reconstruction and accelerated patient recovery.

A Dorsal Arachnoid Web of the Cervical Spine: A Case Report.

Dorsal arachnoid web (DAW) is a rare entity, which has been reported only in the thoracic spine. The authors report the first case of DAW developing in the cervical spine. A 78-year-old man with several-year progressive gait disturbance and bilateral lower-extremity numbness was referred to our hospital on the suspicion of a non-enhancing cystic cervical spinal tumor. Magnetic resonance imaging (MRI) showed a focal indentation along the dorsal surface of the spinal cord at C7 associated with widened cerebrospinal fluid (CSF) space and increased T2-weighted signals in the cord at C5-C7. DAW was suspected, but because of the atypical location for DAW, computed tomography (CT) myelogram was performed and demonstrated an incomplete blockage of the CSF flow at C7 with neither visible abnormal membranes nor a cyst formation. Intraoperative ultrasonography and operative findings revealed that two fluttering membranes disturbed the CSF flow. The pathology of the excised membranes was arachnoid tissues. DAW should be suspected based on the characteristic MRI findings even if the location is in the lower cervical spine. This case seems to support the theory that DAW may be an incomplete or disrupted formation of an arachnoid cyst.

Intradural Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spinal Cord: A Case Report.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, and develops infrequently in the central nervous system. To our knowledge, this is the first case of EHE of the spinal cord. An 85-year-old man presented with about 6-month progressive myelopathy. Magnetic resonance imaging (MRI) demonstrated an oval-shaped intradural extramedullary mass at T10 level with extensive intramedullary edema. A reddish tumor was removed via a total laminectomy of T9-T10. Histologically, the tumor cells with nuclear atypia and active mitoses were immunopositive for vascular tumor markers, and formed a lobular architecture associated with capillary-sized vessels lined with edematous endothelial cells. Although very rare, EHE should be considered in the differential diagnosis of a spinal intradural extramedullary tumor.

Case of Hemorrhagic Moyamoya Disease Associated with Von Willebrand Disease.

BACKGROUND: Association of moyamoya disease (MMD) with von Willebrand disease (vWD) is extremely rare. CASE DESCRIPTION: We first report a 27-year-old female case of MMD concurrent with vWD type 1, which presented as hemorrhagic stroke. The patient underwent a revascularization surgery with perioperative replacement therapy of von Willebrand factor and coagulation factor VIII. No hemorrhagic complications occurred. CONCLUSIONS: The patient overcame postoperative transient neurological events and fully recovered. We discuss appropriate perioperative supplementation of coagulation factors for a revascularization surgery for MMD with vWD.

Tiny Falx Meningioma Causing Massive Interhemispheric Subdural Hematoma: A Case Report.

Bleeding from meningiomas is well known, but massive subdural hemorrhage from a very small meningioma is rare. A 61-year-old woman presented with a sudden-onset headache and slight right hemiparesis without a history of trauma. Computed tomographic scan showed bilateral acute/subacute interhemispheric subdural hematoma, but contrast-enhanced computed tomography (CT) scan, non-enhanced magnetic resonance imaging (MRI) and digital subtraction angiography failed to detect the cause. The hematoma was conservatively treated. Three weeks later, CT scans showed a vestige of the hematoma along the falx. However, repeated angiogram revealed a tumor stain on the falx supplied by the middle meningeal arteries, leading to the tentative diagnosis of meningioma. The tumor was removed and histologically diagnosed as angiomatous meningioma. It is rare that falx meningioma causes massive interhemispheric subdural hematoma, and the diagnosis of the causative lesion is challenging if tumor is small. We review the literature and discuss the characteristics.

Gut hormones of preterm infants with abdominal symptoms and hypothyroxinemia.

BACKGROUND: The pathogenesis of abdominal symptoms in premature infants with hypothyroxinemia is not understood; therefore, we investigated changes in gut hormones before and after levothyroxine sodium (T4-Na) supplementation in preterm infants with abdominal symptoms and hypothyroxinemia. METHODS: In eight preterm study subjects and 14 gestational age-matched controls, fasting serum concentration of leptin, glucose-dependent insulinotropic polypeptide (GIP), glucagon-like peptide-1 (GLP-1), peptide YY (PYY), pancreatic polypeptide, insulin, amylin and ghrelin was measured using a bead array system. RESULTS: Serum GLP-1, GIP and PYY in the subjects before T4-Na supplementation were lower than in controls at age 2 weeks. After improvement of abdominal symptoms and free thyroxine, serum levels of the three gut hormones in the subjects were increased and were not different from those in the control patients. CONCLUSIONS: In preterm infants with abdominal symptoms, serum GLP-1, GIP and PYY might be related to thyroid function.

Gut hormone profiles in preterm and term infants during the first 2 months of life.

AIM: To investigate changes of gut hormones in term and preterm infants in the first 2 months after birth, as the role and relationships of gut hormones in premature infants has not been well elucidated. METHODS: In 29 preterm and five term infants, fasting serum concentrations of leptin, glucose-dependent insulinotropic polypeptide (GIP), glucagon-like peptide-1 (GLP-1), peptide YY (PYY), pancreatic polypeptide, insulin, amylin, and ghrelin were measured using a bead array system. RESULTS: Serum leptin concentration soon after birth showed a positive correlation with gestational week in all infants (r=0.623, p<0.01). Serum leptin level rapidly decreased in all infants. In preterm infants, serum GLP-1 levels at birth showed negative correlations with gestational week (r=-0.447, p=0.02). Serum GIP, GLP-1, and PYY levels increased after birth and were persistently high until 10 weeks of life. CONCLUSION: Serum concentrations of different gut hormones changed postnatally in their specific ways in preterm infants.

Vascular corticobasal syndrome caused by unilateral internal carotid artery occlusion.

A 65-year-old man developed progressive worsening of right-sided limb-kinetic apraxia and extrapyramidal dysfunction. His left internal carotid artery was found to be occluded, and there was general atrophy and severely decreased cerebral blood flow in the left hemisphere. He had experienced an acute infarction in the left watershed area before superficial temporal artery to middle cerebral artery bypass surgery. After surgery, the cerebral blood flow in the left hemisphere was remarkably improved. Unilateral internal carotid artery occlusion may result in clinical manifestations similar to corticobasal degeneration.

[A 68 year-old man presenting ideomotor apraxia and incomplete Gerstmann syndrome with multiple cystic lesions in the left hemisphere].

A 68-year-old man was referred to our hospital with tension-type headaches and a 1-year history of dementia. On neurologic examination, he had ideomotor apraxia and incomplete Gerstmann syndrome that was characterized by acalculia, agraphia, and finger agnosia. On imaging, multiple cystic lesions reported as "unusually dilated perivascular spaces" were observed along the medullary arteries in the left hemisphere; some of them had adjacent hyperintense areas in fluid attenuated inversion recovery images. We assumed that the multiple cystic lesions caused his higher cerebral dysfunction, because ideomotor apraxia and Gerstmann syndrome are usually indicative of a left parietal lobe lesion. MR spectroscopy in the lesion site revealed increased lactate. On MR angiography, the left middle cerebral artery and the left posterior cerebral artery were poorly visualized without localized stenosis. Technetium-99 bicisate single-photon emission computed tomography showed severely decreased cerebral blood flow in the left hemisphere. Electroencephalography showed slow waves in the left hemisphere.

The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases.

Neuronal intranuclear inclusions (NIIs) are the pathological hallmark of polyglutamine (polyQ) diseases. We previously found that the RNA-binding protein FUS/TLS is the major component of nuclear polyQ aggregates of a cellular model of Huntington disease. In this study, we revealed that FUS/TLS binds to NIIs in the human brains from patients with spinocerebellar ataxia type 1, 2, 3, and dentatorubral-pallidoluysian atrophy. Recent reports have revealed that mutations in FUS/TLS gene are responsible for familial amyotrophic lateral sclerosis 6 (ALS6). Our results indicated that changing FUS/TLS to an insoluble form may be a common process in polyQ diseases and ALS6.

Correlation between neurological dysfunction with vitamin E deficiency and gastrectomy.

OBJECTIVE: We previously reported on vitamin E malabsorption after gastrectomy. In this study, we focused on neurological dysfunction due to serum vitamin E decrease during the postgastrectomy period in lager number of patients. METHODS: We examined the type of gastrectomy, type of reconstruction, serum vitamin E level, and neurological status for 96 gastrectomy patients. RESULTS: Low serum vitamin E levels were observed in 20 patients, and 10 of those patients suffered some neurological symptoms, i.e., peripheral neuropathy, limb or truncal ataxia. Vitamin E levels tended to decrease with time after gastrectomy, and the number of patients with low serum vitamin E levels increased at about 50 months after gastrectomy. This relationship was stronger in total gastrectomy patients than in subtotal gastrectomy patients. Ten patients were given oral vitamin E, and serum vitamin E levels normalized in 9 of the patients and neurological abnormalities improved in 8 patients. An oral intake of 300 mg or more of vitamin E was necessary for normalization of vitamin E levels. CONCLUSIONS: Gastrectomy should be considered a risk for vitamin E deficiency and neurological disturbance over the long-term clinical course. An oral vitamin E supply can improve serum vitamin E levels and neurological symptoms.

Vitamin E malabsorption and neurological consequences after gastrectomy for gastric cancer.

BACKGROUND/AIMS: In order to clarify vitamin E malabsorption after gastric surgery, we evaluated serum vitamin E level and neurological consequences in patients who had undergone gastrectomy for gastric cancer. METHODOLOGY: We studied forty-eight patients (30 men, 18 women) with a mean age of 68.0 years, who had previously undergone gastrectomy for gastric cancer and had no evidence of recurrence. The types of operation consisted of subtotal gastrectomy in 26 patients and total gastrectomy in 22 patients. We measured postoperative body weight, white blood cells, red blood cells, hemoglobin, hematocrit, platelets, and serum levels of vitamins E, vitamin B12, folic acid, total cholesterol, triglycerides, total protein, and albumin. RESULTS: Serum vitamin E level was decreased in 7 (14.6%) of the 48 patients. The occurrence of low vitamin E level was significantly more frequent in the total gastrectomy group than in the subtotal gastrectomy group. In relation to reconstruction procedures, the incidence of low vitamin E level was significantly higher in patients without food passage through the duodenum. The low vitamin E level was significantly associated with low total cholesterol level. The incidence of neuropathy was significantly higher in patients with low vitamin E level. CONCLUSIONS: We assume that vitamin E deficiency more likely occurs after the reconstruction procedure in which food does not pass through the duodenum. Transport disturbance with loss of passage through the duodenum consequently may be the major cause of malabsorption. Differential diagnosis of neurological symptoms in post-gastrectomy patients should include hypovitaminosis E.